CASE REPORT Open Access

Neonatal respiratory distress syndromerevealing a cervical bronchogenic cyst: a case reportPenelope Thaller1, Catherine Blanchet2, Maliha Badr1, Renaud Mesnage1, Nicolas Leboucq3, Michel Mondain2

and Gilles Cambonie1*

Abstract

Background: Bronchogenic cyst is a congenital malformation, rarely located in the cervical region and almostnever involved in a neonate with acute respiratory distress in the delivery room.

Case presentation: A female newborn with respiratory distress syndrome caused by a large left cervical mass.Intubation was difficult due to tracheal deviation. Magnetic resonance imaging confirmed a left cervical cystdisplacing the trachea and esophagus laterally. Surgical excision was performed via a cervical approach on the 5thday, and pathological examination revealed a bronchogenic cyst. The patient's course was complicated by left vocalcord paralysis and necrotic lesions in the glottic and subglottic regions; she required a tracheostomy on the 13thday. Inflammatory stenosis in the subglottic region required balloon dilation once, 20 days later. Proximal esophagealstenosis induced transient upper airway obstruction with salivary stasis. Decannulation was performed at 2 months andthe patient was discharged 10 days later.

Conclusion: A bronchogenic cyst can exceptionally obstruct the airways in the neonatal period. Surgical excision isnecessary, but postoperative complications may occur if the cyst is in close contact with the trachea and esophagus,including necrotic and stenotic lesions of the upper aerodigestive tract. In those situations, tracheostomy may benecessary for mechanical ventilation weaning and the initiation of oral feeding.

Keywords: Cervical bronchogenic cyst, Neonatal respiratory distress syndrome, Tracheostomy

BackgroundBronchogenic cyst is a congenital malformation of thetracheobronchial tree. It is usually located in the medias-tinum or lung parenchyma, though rarely can be seen inextrathoracic locations such as the neck. In 2004, Mehtaet al. reported a series of 4 cases of cervical broncho-genic cysts over a 22-year period, with the age of discov-ery ranging from 3 weeks to 6 years [1]. In 2008,Teissier et al. reported 8 cases over a period of 13 years[2]. The signs and symptoms are variable, but usuallyconsistent with a neck mass associated with chroniccough or stridor [3]. Some cases are not diagnosed untiladulthood, when an isolated laterocervical mass is dis-covered incidentally [4]. We report an exceptional case

of cervical bronchogenic cyst revealed by acute respira-tory distress in the neonatal period.

Case presentationThe patient’s mother received prenatal care during preg-nancy, with 3 antenatal US exams showing normal fetaldevelopment. Labor was induced at 41+4 gestationalweeks in a type 2a maternity ward and delivery was bycesarean section because of abnormal fetal heart rate.The newborn was female with a birth weight of 3045 g.The newborn showed ineffective ventilation from thefirst postnatal minute, and positive pressure ventilationwith a bag and mask was immediately started. A left cer-vical swelling was also observed. Respiratory distress,with suprasternal tugging and stridor, occurred at everyattempt to withdraw ventilatory support. Furthermore,FiO2 of 90 % was required to maintain SpO2 above90 %. Two attempts of orotracheal intubation failed be-cause the larynx could not be visualized. A more experi-enced pediatrician was called for help. Standard direct

* Correspondence: g-cambonie@chu-montpellier.fr1Department of Neonatology and Pediatric Intensive Care Unit, HôpitalArnaud de Villeneuve, 371 Avenue du Doyen Gaston Giraud, 34295Montpellier Cedex 5, FranceFull list of author information is available at the end of the article

© 2015 Thaller et al. This is an Open Access article distributed under the terms of the Creative Commons Attribution License(http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium,provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Thaller et al. BMC Pediatrics (2015) 15:72 DOI 10.1186/s12887-015-0363-2

laryngoscopy found a right laryngeal deviation with a re-stricted laryngeal orifice. After sedation with midazolam(0.1 mg/kg intravenous, 2 doses 10 min apart), trachealintubation was achieved at the 3rd attempt, with a 2.5-mm endotracheal tube, at 1 h 30 min after birth. Thenewborn was then transferred to a type 3 neonatal unit.On admission, the newborn was ventilated in assist-

control pressure mode with FiO2 set at 21 %. Clinicalexamination confirmed a noninflammatory cervical swell-ing that was soft on palpation. Chest x-ray revealed theendotracheal tube deflected to the right with respect tothe spinal axis. Cervical US showed a thin-walled cysticmass measuring 24 × 28 × 37 mm displacing the tracheaand the left thyroid lobe medially and forward, and thecarotid and jugular vessels laterally and posteriorly. MRIidentified a large cystic mass with well-defined walls thatlaterally displaced the aerodigestive tract (Fig. 1).In the first 48 h of management, peak inspiratory pres-

sures > 25 cm H2O were necessary to observe chest ex-pansion and hear vesicular breath sounds at auscultation.Volume mode was tested, but it also generated high in-spiratory pressures, between 25 and 30 cm H2O, despitethe selection of a minimal tidal volume (5 ml/kg) and theuse of permissive hypercapnia (PCO2 allowed to rise ashigh as 60 mmHg). On the assumption that the cyst mightbe squeezing the endotracheal tube, we performed fineneedle aspiration of the cyst in the operating room, undergeneral anesthesia, on postnatal day 3. Twenty millilitersof clear liquid was removed for cytological analysis, whichrevealed both respiratory-type epithelium and squamousepithelium. Rigid bronchoscopy performed at the sametime confirmed tracheal compression and no laryngealfistula. A 3-mm endotracheal tube was put into place tosecure the airway. On day 5, surgery using a cervical ap-proach isolated a unilocular cystic structure measuring25 × 23 × 6 mm in close contact with the trachea and

esophagus. Dissection was not possible from the tracheaor the esophagus, and limited resection of trachea andesophagus was therefore mandatory, resulting in anopening of both structures. Both were then sutured.Histological examination showed that the cyst wall wascomposed of fibrous and smooth fibromuscular tissue,with some seromucinous bronchial gland cells. The wallwas lined by a layer of respiratory-type ciliated cells. Thesefindings suggested the diagnosis of bronchogenic cyst.After the first attempt at extubation on the day after

surgery, the infant had respiratory distress due to laryn-gotracheal edema and left vocal fold paresis that was re-fractory to treatment with nebulized epinephrine andcorticosteroids and required reintubation. The secondattempt at 8 days postsurgery was equally unsuccessful.The upper respiratory tract was explored under generalanesthesia at a postnatal age of 13 days. We observededema of the left laryngeal ventricle and the anteriorthird of the glottis, associated with the necrotic appear-ance of the mucosa in the left lateral subglottis (Fig. 2).Such lesions in a newborn who could not be weanedfrom mechanical ventilatory support prompted immedi-ate tracheostomy.Sedation was gradually reduced over 48 h postsurgery,

and mechanical ventilation was ended 2 weeks later withthe infant ventilating effectively with the tracheal cannula.Laryngotracheobronchoscopy under general anesthesia

on day 33 revealed a subglottic stenosis treated with a6-mm diameter balloon dilation. Proximal esophagealstenosis was also observed and not treated. Videographicexploration of swallowing confirmed the images of a veryproximal partial esophageal stenosis, from C3 to C4.After a final endoscopic assessment at 2 months show-

ing the absence of airway stenosis, the cannula was with-drawn. The infant was then discharged 10 days later.She was exclusively breastfed and weight and height

AB

T1

A

BT2

Fig. 1 Cervical thoracic MRI. Coronal T1-weighted sequence showing a voluminous left laterocervical mass displacing the trachea (a) and carotid-jugularaxis (b). Axial T2-weighted sequence showing tracheal compression (a) and esophageal compression (b) by the cyst

Thaller et al. BMC Pediatrics (2015) 15:72 Page 2 of 4

gains were satisfactory, with respective gains of 915 gand 7 cm since birth, with absence of swallowing diffi-culty in relation to the partial esophageal stenosis.

DiscussionThe respiratory bud develops from the ventral side ofthe primitive foregut from the 4th week of development.Cervical bronchogenic cyst may result from an abnormaldivision of the bud and migration to an ectopic localizationin the neck region [1,5,6]. Other extrathoracic localizationshave been described, including subcutaneous [7] andeven abdominal [8] sites.Antenatal discovery of a bronchogenic cyst is rare and

localization is generally intrathoracic [9,10]. One casehas been reported of a cervico-mediastinal bronchogeniccyst detected by a cervical mass on prenatal US [11].Undetected cervical bronchogenic cyst may also causepolyhydramnios by esophageal compression [12]. In boththis case and ours, an abnormal fetal heart rate precipi-tated delivery by cesarean section. Nevertheless, thedirect and indirect impact of this malformation on dis-turbances in fetal monitoring is difficult to establish.The immediate neonatal respiratory distress of our pa-

tient was explained by direct compression of the trachea,causing major difficulties for intubation because thelarynx could not be visualized. The use of a video-laryngoscope for intubation can improve the viewingconditions of the upper airways, making it easier toidentify the laryngeal inlet. Rigid bronchoscopy or aflexometallic tube can also relieve glottic-subglotticcompression, although great caution is required whileadvancing the scope or the tube. The ex utero intrapar-tum treatment (EXIT) procedure can also be done ifprenatal diagnosis suggests fetal airway obstruction by aneck mass. Its use in this context has mainly been

reported for cervical lymphatic malformations andcervical teratomas [13,14].The main differential diagnosis is cystic hygroma,

which is more likely in the setting of a multilocular cyst,and more unlikely in the setting of a unilocular cyst.Other differential diagnoses include thymic cyst, bran-chial cyst and thyroglossal duct cyst, but they are rarelyresponsible for neonatal respiratory distress. Only histo-logical examination of the resected specimen confirmsthe diagnosis.MRI is essential before surgery, as it provides the sur-

geon with precise information on the anatomical rela-tionships with adjacent organs [15]. Rigid bronchoscopyassesses the degree of cyst-trachea contact and the needfor tracheal surgery [12]. Needle aspiration of the cystusually has low diagnostic value [16]. It was performedin our patient to temporarily reduce the tracheal com-pression. However, neither cyst aspiration nor the largercaliber endotracheal tube introduced following bron-choscopy significantly reduced peak inspiratory pres-sures, which remained high until surgery.The only treatment is surgical excision, even for an

asymptomatic cyst, given the risks of infection, hemorrhageand, rarely, malignant transformation [17]. The paralysis ofthe left vocal cord may have resulted from direct injury tothe laryngeal nerve during surgery. Paralysis was reportedin two other cases [2,12], but in one of these cases thiscondition had already been observed during the preopera-tive bronchoscopy, suggesting direct mechanical interfer-ence between the cyst and the ipsilateral laryngeal nerve[2]. Evaluation of vocal cord mobility before surgery wasnot possible in our case because of the depth of sedationduring bronchoscopy.Several factors probably contributed to the alterations

in the glottic and subglottic regions observed after the

A B

Glottis Sub-glottis

Fig. 2 Postoperative endoscopy. Glottic region: anterior synechia (a) causing luminal stenosis of 50 %. Subglottic region: sutured tracheal wound(b) and inflammatory alterations causing grade 1 stenosis

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intervention, including intubation and the repeated at-tempts to intubate, the inflammation associated with la-ryngeal surgery, and the prolonged mechanical ventilation.In a newborn, edemic and necrotic lesions further narrowan already physiologically narrow larynx, which is anadditional motivation to perform tracheostomy.In addition to the vocal cord paralysis, persistent air-

way obstruction after laryngeal surgery should alwaysraise the suspicion of a local cause, such as stenosis. Inour patient, a proximal esophageal stenosis was alsopresent after surgery. The damage to the esophageal mu-cosa was probably caused by the surgery, secondary tocyst resection and the suturing of borders. This stenosis,however, had only transient and moderate clinical conse-quences and did not preclude satisfactory breastfeeding,even with the tracheal cannula.

ConclusionCervical bronchogenic cyst is rare and diagnosis is mostoften made in childhood. However, it should be systemat-ically considered in all cases of neonatal cervical tumor,especially if respiratory distress is present. Prenatal diag-nosis may be guided by an unexplained polyhydramniosand/or direct visualization of a cyst, in which case deliveryshould be performed in a maternity hospital with a type 3neonatal unit. The EXIT procedure, with a multidisciplin-ary team approach, should also be planned for fetuses withevidence of airway obstruction on prenatal images.

ConsentWritten informed consent was obtained from the par-ents of the patient for publication of this case report andthe accompanying images. A copy of the written consentis available for review by the Editor of this journal.

AbbreviationsUS: Ultrasound; FiO2: Fraction of inspired oxygen; SpO2: Oxygen saturation bypulse oximetry; MRI: Magnetic resonance imaging; cm H2O: centimeter of water(0.7355 mmHg); PCO2: Partial pressure of carbon dioxide measured on capillaryblood gas sampling (mmHg); C3: 3rd cervical vertebra; C4: 4th cervical vertebra.

Competing interestsThe authors declare that they have no competing interests.

Authors’ contributionsPT reviewed the literature and prepared the manuscript, CB helped to draftthe manuscript, MB and RM made a critical reading of the manuscript andparticipated to revisions, NL helped to draft the manuscript, MM helped todraft the manuscript and participated to revisions, GC reviewed the literature,prepared the manuscript and is the corresponding author. All authors readand approved the final version of the manuscript.

AcknowledgementThe authors thank Mrs C Stott-Carmeni for reviewing the manuscript and foreditorial assistance.This case study is from the Neonatal Intensive Care Unit, Arnaud deVilleneuve Hospital, Montpellier University Hospital Center.

Author details1Department of Neonatology and Pediatric Intensive Care Unit, HôpitalArnaud de Villeneuve, 371 Avenue du Doyen Gaston Giraud, 34295Montpellier Cedex 5, France. 2Department of Pediatric Otorhinolaryngology,Hôpital Arnaud de Villeneuve, 371 Avenue du Doyen Gaston Giraud, 34295Montpellier Cedex 5, France. 3Department of Neuroradiology, CHUMontpellier, F-34000 Montpellier, France.

Received: 17 October 2014 Accepted: 13 April 2015

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